Frambu Resource Centre for Rare Disorders (Frambu), Siggerud, Norway.
National Neuromuscular Centre (NMK), University Hospital of North Norway, Norway.
J Neuromuscul Dis. 2021;8(4):457-468. doi: 10.3233/JND-200604.
Primary periodic paralysis (PPP) are rare inherited neuromuscular disorders including Hypokalemic periodic paralysis (HypoPP), Hyperkalemic periodic paralysis (HyperPP) and Andersen-Tawil syndrome (ATS) characterised by attacks of weakness or paralysis of skeletal muscles. Limited effective pharmacological treatments are available, and avoidance of lifestyle related triggers seems important.
Our aim was to search and assess the scientific literature for information on trigger factors related to nutrition and physical activity in PPP.
We searched Ovid Medline and Embase database for scientific papers published between January 1, 1990, to January 31, 2020.
We did not identify published observation or intervention studies evaluating effect of lifestyle changes on attacks. Current knowledge is based on case-reports, expert opinions, and retrospective case studies with inadequate methods for description of nutrition and physical activity. In HypoPP, high carbohydrate and salt intake, over-eating, alcohol, dehydration, hard physical activity, and rest after exercise are frequently reported triggers. Regarding HyperPP, fasting, intake of potassium, alcohol, cold foods or beverages, physical activity, and rest after exercise are frequently reported triggers. No nutrition related triggers are reported regarding ATS, exercise can however induce ventricular arrhythmias.
Our results support that dietary intake and physical activity may play a role in causing paralytic attacks in PPP, although the current scientific evidence is weak. To provide good evidence-based patient care, several lifestyle aspects need to be further assessed and described.
原发性周期性瘫痪（PPP）是罕见的遗传性神经肌肉疾病，包括低钾周期性瘫痪（HypoPP）、高钾周期性瘫痪（HyperPP）和 Andersen-Tawil 综合征（ATS），其特征为骨骼肌无力或瘫痪发作。目前可获得的有效药物治疗有限，避免与生活方式相关的诱因似乎很重要。
我们旨在搜索和评估 PPP 相关营养和体力活动的诱因的科学文献信息。
我们在 Ovid Medline 和 Embase 数据库中检索了 1990 年 1 月 1 日至 2020 年 1 月 31 日期间发表的科学论文。
我们未发现评估生活方式改变对发作影响的观察或干预研究。目前的知识基于病例报告、专家意见和回顾性病例研究，对营养和体力活动的描述方法不充分。在 HypoPP 中，高碳水化合物和盐的摄入、暴饮暴食、酒精、脱水、剧烈体力活动以及运动后休息，这些都是常见的诱发因素。对于 HyperPP，禁食、钾摄入、酒精、冷食或饮料、体力活动以及运动后休息，这些都是常见的诱发因素。关于 ATS，没有报道与营养相关的诱因，但运动可能会引起室性心律失常。
我们的研究结果表明，饮食摄入和体力活动可能在 PPP 的瘫痪发作中起作用，尽管目前的科学证据很薄弱。为了提供良好的基于证据的患者护理，还需要进一步评估和描述几个生活方式方面。
